Pulmonary hypertension screening
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assistant Editor(s)-in-Chief: Ralph Matar
Overview
Patients with a known BMPR2 mutation, scleroderma, and portal hypertension undergoing evaluation for liver transplantation should receive periodic screening for pulmonary hypertension (PH) through a thorough assessment of the presence of symptoms, physical examination, chest X ray, electrocardiography, and echocardiogram. Additional investigation with right heart catheterization should be performed if screening is suggestive of the presence of PH.[1]
Screening
Shown below is a table summarizing the recommended screening in several medical conditionas associated with elevated risk for PH.[2]
Condition | Recommended screening |
Known BMPR2 mutation | Echocardiogram (yearly) |
BMPR2 mutation in a first degree relative | Genetic counseling BMPR2 genotyping |
Family history for PAH in 2 or more relatives | Genetic counseling BMPR2 genotyping |
Systemic sclerosis | Echocardiogram (yearly) |
Portal hypertension | Echocardiogram if orthotopic liver transplantation is in consideration |
Sickle cell disease | Echocardiogram (yearly) |
Previous use of fenfluramine | Echocardiogram in case of symptoms |
Congenital heart disease | Echocardiogram at the time of diagnosis |
These are the parameters that should be evaluated on Echocardiogram:
- Right atrial and right ventricular enlargement.
- Reduced right ventricular function.
- Displacement of the interventricular septum.
- Tricuspid regurgitation(TR).
- Tei index or myocardial performance index (measured by Doppler ultrasound).
- Pericardial effusion (presence also indicates a higher mortality rate).
The Tei index is an index of combined right ventricular systolic and diastolic function obtained by dividing the sum of both isovolumetric contraction and relaxation intervals by the ejection time.